Scaphocephaly, or sagittal craniosynostosis, is a type of cephalic disorder which occurs when there is a premature fusion of the sagittal suture. Premature closure results in limited lateral expansion of the skull resulting in a characteristic long, narrow head.[1] The skull base is typically spared.[2]
Scaphocephaly is the most common of the craniosynostosis conditions and accounts for approximately 50% of all craniosynostosis.[3] It is most commonly idiopathic (non-syndromic).
Etiologyedit
Non-syndromic
The underlying cause of the non-syndromic form is unknown. Over 100 mutations have been associated, including mutations in the FGFR genes.[4] Several potential risk factors have been identified for craniosynostosis include:[5]
Advanced maternal age
White maternal race
Maternal smoking
Male infant
Certain paternal occupations (e.g. agriculture, forestry, repairmen) [6]
Syndromic
Sagittal craniosynostosis is seen in many conditions and syndromes:[7]
Diagnosis of scaphocephaly is with physical exam, which may show characteristic features such as an elongated head in the anterior-posterior dimension, narrow head in the lateral dimension, and bony ridge at the vertex.[8]
Further evaluation with imaging may also be performed. Ultrasound may be used to detect fusion of the suture. CT scans may also be used to help with surgical planning and to diagnose associated hydrocephalus, which has been found to be present in 44% of cases in one study.[9] A measure of cephalic index may also be reduced, however the reliability of measurements may not be a reliable measure.[10]
Classificationedit
Scaphocephaly can be classified into specific types, depending on morphology, position, and suture closure:[11]
Bathrocephaly– bulging of the mid-section of the occipital bone; also associated with isolated mendosal suture synostosis.[12]
Clinocephaly– flat cranium due to loss of cranial convexity;[13] top of head is depressed inwards.[14]
This condition can be corrected by surgery if the child is young enough, typically within the first 3–6 months.[8] The goal of treatment is to correct intracranial pressure and repair bony deformities.[18] The decision to treat is multifactorial and should be performed at a center with an experienced craniofacial team.[19] In addition to the primary craniofacial surgeon, team members may include audiologists, dentists, otolaryngologists, neurosurgeons, plastic surgeons, and other supporting members.[19]
Surgery is generally aimed at removal of the fused sagittal suture to allow for lateral expansion of the skull. Surgical options include:[8]
Endoscopic strip craniectomy: Minimally invasive removal of the fused suture. Following surgery, patients typically wear a helmet to help shape the head. Helmets are usually worn for 3–12 months.[20]
Open cranial vault remodeling: Open surgical removal of the fused sagittal suture and re-shaping of the skull, generally with resorbable plates.[21]
Spring cranioplasty: Combination of an endoscopic strip craniectomy with placement of springs which provide continuous force for re-shaping the skull.[22]
Terminologyedit
The term, from Greek skaphe meaning 'light boat or skiff' and kephale meaning 'head', describes a specific shape of a long narrow head[23] that resembles a boat.
^Ye, Xiaoqian; Guilmatre, Audrey; Reva, Boris; Peter, Inga; Heuzé, Yann; Richtsmeier, Joan T.; Fox, Deborah J.; Goedken, Rhinda J.; Jabs, Ethylin Wang; Romitti, Paul A. (2016-03-01). "Mutation Screening of Candidate Genes in Patients with Nonsyndromic Sagittal Craniosynostosis". Plastic and Reconstructive Surgery. 137 (3): 952–961. doi:10.1097/01.prs.0000479978.75545.ee. ISSN 1529-4242. PMC4770826. PMID 26910679.
^Kabbani, Haidar; Raghuveer, Talkad S. (2004-06-15). "Craniosynostosis". American Family Physician. 69 (12): 2863–2870. PMID 15222651.
^Bradley, C. M.; Alderman, B. W.; Williams, M. A.; Checkoway, H.; Fernbach, S. K.; Greene, C.; Bigelow, P. L.; Reif, J. S. (1995-05-01). "Parental occupations as risk factors for craniosynostosis in offspring". Epidemiology. 6 (3): 306–310. doi:10.1097/00001648-199505000-00020. ISSN 1044-3983. PMID 7619941. S2CID 24625014.
^ abcSandoval, Jose I.; De Jesus, Orlando (2022), "Scaphocephaly", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 33620830, retrieved 2023-01-03
^Wall, Steven A.; Thomas, Gregory P. L.; Johnson, David; Byren, Jo C.; Jayamohan, Jayaratnam; Magdum, Shailendra A.; McAuley, David J.; Richards, Peter G. (2014-12-01). "The preoperative incidence of raised intracranial pressure in nonsyndromic sagittal craniosynostosis is underestimated in the literature". Journal of Neurosurgery. Pediatrics. 14 (6): 674–681. doi:10.3171/2014.8.PEDS1425. ISSN 1933-0715. PMID 25259602.
^Fearon, Jeffrey A.; Ditthakasem, Kanlaya; Herbert, Morley; Kolar, John (2017-07-01). "An Appraisal of the Cephalic Index in Sagittal Craniosynostosis, and the Unseen Third Dimension". Plastic and Reconstructive Surgery. 140 (1): 138–145. doi:10.1097/PRS.0000000000003422. ISSN 1529-4242. PMID 28654600. S2CID 31253312.
^Skadorwa, Tymon; Wierzbieniec, Olga (August 5, 2022). "The foramen magnum in scaphocephaly". Child's Nervous System. 38 (11): 2163–2170. doi:10.1007/s00381-022-05624-2. PMC9617951. PMID 35931858.
^Neto, Antonio Rodrigues de Aguiar. "Bathrocephaly | Radiology Reference Article | Radiopaedia.org". Radiopaedia. Retrieved 2023-04-06.
^ abKim, Hyun; Roh, Hong; Lee, Il (May 2016). "Craniosynostosis : Updates in Radiologic Diagnois - PMC". Journal of Korean Neurosurgical Society. 59 (3): 219–226. doi:10.3340/jkns.2016.59.3.219. PMC4877543. PMID 27226852.
^Pfaff, Miles; Fenton, Regina; Goldstein, Jesse (2023). "The Clinical Significance of Clinocephaly in Late-Presentation Sagittal Craniosynostosis". The Cleft Palate Craniofacial Journal. 60 (5): 521–525. doi:10.1177/10556656211064484. PMID 35538850. S2CID 248695057. Retrieved April 5, 2023.
^"sphenocephaly", The Free Dictionary, retrieved 2023-04-06
^Ciurea, AV; Toader, C; Mihalache, C (2011-11-14). "Actual concepts in scaphocephaly". Journal of Medicine and Life. 4 (4): 424–431. ISSN 1844-122X. PMC3227159. PMID 22514579.
^ abBuchanan, Edward P; Xue, Yunfeng; Xue, Amy S; Olshinka, Asaf; Lam, Sandi (2017-07-06). "Multidisciplinary care of craniosynostosis". Journal of Multidisciplinary Healthcare. 10: 263–270. doi:10.2147/JMDH.S100248. ISSN 1178-2390. PMC5505551. PMID 28740400.